Management and Treatment of Pediatric Spinal Cord Ependymoma: Results from an International Multi-Institutional Review

Abstract PURPOSE Pediatric Spinal cord ependymoma (SCE) is rare, and the management is often heterogeneous across centers. We evaluated the impact of clinical, pathologic, and treatment-related factors on outcomes in a multi-institutional, international cohort. METHODS SCE patients age <21 years were reviewed across 5 institutions. We utilized nonparametric descriptive statistics, survival, and recursive partitioning analysis (RPA) to examine patient, tumor, histopathologic and treatment characteristics, failure pattern, and cause of death. RESULTS 125 patients were identified, 18 (14.4%) with metastases. Initial surgery was GTR, and STR in 44, 56% of patients respectively. Histology was grade 1, 2, and 3 in 55, 17.7 and 23.2% respectively. 55 patients with initial GTR were observed (52.7%) or irradiated (43.6%); 60 patients had STR and were observed (40%) or irradiated (60%). The 7-year event-free (EFS) and overall survival (OS) was 60% (95% CI 51.5–71.4) and 79% (95% CI 71.1–87.8) respectively. STR and metastasis increased the hazard for death [HR 1.87, 95% CI 1.02–3.57, p=0.05 (vs. GTR)] and [HR 2.28, 95% CI 1.1–5.2, p=0.048 (vs. localized)] respectively. Across 43 failures, local failure predominated (48.8%). Distant and combined failure occurred in 30.2 and 13.9% respectively. Adjuvant RT offered a 20% absolute improvement (vs. observation) in EFS at 5 years regardless of extent of resection. RPA identified thoracic (vs. non-thoracic), grade (1 & 3 vs. 2), STR (vs. GTR) and metastases as determinants of inferior EFS. CONCLUSIONS Tumor and treatment-related factors are predictive of EFS. OS is favorable despite diverse schema and frequent distant failures.

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Microsurgical resection of intramedullary spinal cord ependymoma

Neurosurgical FOCUS ◽

10.3171/2014.v3.focus14276 ◽

2014 ◽

Vol 37 (v2supplement) ◽

pp. Video9

Author(s):

Paul C. McCormick

Keyword(s):

Spinal Cord ◽

Growth Rate ◽

Spinal Cord Tumor ◽

Intramedullary Spinal Cord ◽

Clear Margin ◽

Intramedullary Spinal Cord Tumor ◽

Link Type ◽

Microsurgical Resection ◽

Spinal Cord Ependymoma ◽

Posterior Midline

Ependymomas are the most commonly occurring intramedullary spinal cord tumor in adults. With few exceptions these tumors are histologically benign, although they exhibit some biologic variability with respect to growth rate. While unencapsulated, spinal ependymomas are non-infiltrative and present a clear margin of demarcation from the surrounding spinal cord that serves as an effective dissection plane. This video demonstrates the technique of microsurgical resection of an intramedullary ependymoma through a posterior midline myelotomy.The video can be found here: http://youtu.be/lcHhymSvSqU.

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Resection of a Large Thoracic Schwannoma: 2-Dimensional Operative Video

Operative Neurosurgery ◽

10.1093/ons/opaa381 ◽

2020 ◽

Author(s):

Kyle B Mueller ◽

Jean-Marc Voyadzis

Keyword(s):

Spinal Cord ◽

Case Reports ◽

Intraoperative Ultrasound ◽

Neurofibromatosis Type ◽

High Yield ◽

Peripheral Nerve Sheath Tumors ◽

Thoracic Level ◽

Institutional Review ◽

Patient Consent ◽

Intradural Extramedullary

Abstract Spinal schwannomas most likely occur at the thoracic level and within the intradural extramedullary compartment. They are benign, typically slow-growing, peripheral nerve sheath tumors that produce symptoms by displacing or compressing the nerve roots and spinal cord. There is an association with patients that have neurofibromatosis type 2. Surgical pearls including the utilization of intraoperative ultrasound for localization, D wave monitoring, and microsurgical dissection are demonstrated. Pertinent high-yield radiographic and histological features of schwannomas are reviewed.1-4 We report the case of a 59-yr-old female who presented with progressively worsening gait instability that was associated with lower extremity numbness progressing to weakness. She had myelopathic findings on examination, which included brisk patellar reflexes and persistent clonus with sensory changes to the umbilicus and mild leg weakness. Full body examination revealed no stigmata of neurofibromatosis. Magnetic resonance imaging of the neuroaxis demonstrated a large, intradural extramedullary mass with peripheral enhancement that spanned the T9 to T11 vertebral levels with severe compression of the spinal cord. There were no intracranial, cervical, or lumbar findings. Surgical intervention was planned with the following objectives: decompression of the neural elements, curative resection, and diagnosis. Patient consent for the procedure was obtained. Institutional Review Board approval for solitary case reports are not needed at our institution.

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EPCO-01. MOLECULAR PROFILING OF SPINAL CORD EPENDYMOMA

Neuro-Oncology ◽

10.1093/neuonc/noab196.000 ◽

2021 ◽

Vol 23 (Supplement_6) ◽

pp. vi1-vi1

Author(s):

Erika Yamazawa ◽

Shota Tanaka ◽

Genta Nagae ◽

Takayoshi Umeda ◽

Taijun Hana ◽

...

Keyword(s):

Spinal Cord ◽

Dna Methylation ◽

Methylation Status ◽

Neurofibromatosis Type ◽

Methylation Pattern ◽

Who Grade ◽

Spinal Ependymoma ◽

Fresh Frozen ◽

Spinal Cord Ependymoma ◽

The Difference

Abstract BACKGROUND Ependymomas are currently classified into 9 subgroups by DNA methylation profiles. Although spinal cord ependymoma (SP-EPN) is distinct from other tumors, diversity within SP-EPN is still unclear. Here, we used transcriptomic and epigenomic profiles to investigate the diversity among Japanese SP-EPN cases. MATERIALS AND METHODS We analyzed 57 SP-EPN patients (32 males and 25 females, aged from 18 to 78 years, median: 52), including two cases of neurofibromatosis type 2, five cases of grade 3 (WHO grade). We obtained transcriptome (RNA-seq) and DNA methylation (Infinium Methylation EPIC array) data from fresh frozen specimens of SP-EPN resected at the University of Tokyo Hospital and our collaborative groups. RESULTS Three cases had a previous intracranial ependymoma operation. Hierarchical clustering of the DNA methylation data showed that these three cases of intracranial origin as a different cluster from spinal origin. The 45 grade 2 spinal ependymoma showed a relatively homogenous methylation pattern. However, the methylation status of HOX gene cluster regions is compatible with the segment of origin, which reflects the cells of origins are derived after the determination of segment identity. RNA sequencing of 57 cases revealed two subgroups within grade 2. Gene ontology analysis of differentially expressed genes suggested the difference in metabolic state such as rRNA translation and mitochondrial respiration between the two expression subgroups. CONCLUSION Epigenetic analysis indicated the accurate body segment origin of SP-EPN. We observed that metabolic states could divide grade 2 spinal cord ependymoma into 2 subgroups and will present the relationship to clinicopathological information.

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Spinal cord ependymoma: a positron emission tomographic study with ( 11 C-methyl)-L-methionine

Neuroradiology ◽

10.1007/s002340050195 ◽

1995 ◽

Vol 38 (1) ◽

pp. 53-55

Author(s):

T. Sasajima ◽

K. Mineura ◽

Y. Itoh ◽

M. Kowada ◽

J. Hatazawa ◽

...

Keyword(s):

Spinal Cord ◽

Positron Emission Tomographic ◽

Positron Emission ◽

Spinal Cord Ependymoma

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Spinal cord ependymoma in two young dogs

Veterinary Quarterly ◽

10.1080/01652176.1988.9694172 ◽

1988 ◽

Vol 10 (3) ◽

pp. 205-210 ◽

Cited By ~ 8

Author(s):

H. R. de Vries‐Chalmers Hoynk van Papendr ◽

J. H. Vos ◽

J. J. van Nes

Keyword(s):

Spinal Cord ◽

Spinal Cord Ependymoma

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Cervical 7-Thoracic 3 Laminoplasty for Resection of Arteriovenous Malformation: 2-Dimensional Operative Video

Operative Neurosurgery ◽

10.1093/ons/opz304 ◽

2019 ◽

Vol 18 (1) ◽

pp. E3-E4

Author(s):

Benjamin K Hendricks ◽

Robert F Spetzler

Keyword(s):

Spinal Cord ◽

Video Recording ◽

Mass Effect ◽

Feeding Artery ◽

Pia Mater ◽

Institutional Review ◽

Signal Change ◽

Spinal Arteriovenous Malformations ◽

Intradural Extramedullary ◽

Vascular Steal

Abstract Symptomatic spinal arteriovenous malformations (AVMs) are most frequently associated with hypoperfusion of the spinal cord, either from venous congestion or vascular steal, and are less frequently associated with hemorrhage. This patient had a large cervicothoracic spinal AVM and presented with right hemibody sensory deficit with intact motor function. The AVM had significant preoperative mass effect on the dorsal spinal cord with cord signal change. Preoperative digital subtraction angiography demonstrated a left supreme intercostal feeding artery and left thyrocervical feeding artery, which was embolized preoperatively. A laminoplasty was performed from cervical 7 to thoracic 3 to allow for adequate visualization. The lesion demonstrated an intradural extramedullary presence, which made preservation of the pia mater paramount during the resection. The AVM was disconnected and removed in its entirety as determined by operative visualization and postoperative imaging. The patient gave informed consent for surgery and video recording. Institutional review board approval was deemed unnecessary. Used with permission from Barrow Neurological Institute, Phoenix, Arizona.

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296. Spinal cord ependymoma: A Retrospective study of 12 patients

Reports of Practical Oncology & Radiotherapy ◽

10.1016/s1507-1367(03)70779-2 ◽

2003 ◽

Vol 8 ◽

pp. S337

Author(s):

R. Zaucha ◽

K. Sosińska-Mielcarek ◽

I. Zander ◽

J. Jassem

Keyword(s):

Spinal Cord ◽

Retrospective Study ◽

Spinal Cord Ependymoma

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Intramedullary Spinal Cord Ependymoma

Neurosurgery ◽

10.1097/00006123-199902000-00014 ◽

1999 ◽

Vol 44 (2) ◽

pp. 269-269

Author(s):

Kintomo Takakura

Keyword(s):

Spinal Cord ◽

Intramedullary Spinal Cord ◽

Spinal Cord Ependymoma

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The cryoprobe retractor in the microsurgical dissection of tumors

Journal of Neurosurgery ◽

10.3171/jns.1978.49.1.0143 ◽

1978 ◽

Vol 49 (1) ◽

pp. 143-145 ◽

Cited By ~ 2

Author(s):

John I. Moseley ◽

Robert W. Rand

Keyword(s):

Spinal Cord ◽

Operating Microscope ◽

Content Type ◽

Spinal Cord Ependymoma ◽

Fine Print

✓ A technique is outlined for retraction of tumors under the operating microscope using the microcryoprobe. The method depends on temperature settings in the −20° to −30° C range to produce a small ice bond uniting tumor and cryoprobe. No attempt is made to create a solid frozen tumor. The locally avascular field and retraction provided ideal circumstances for microdissection. Examples of this approach are outlined with case summaries for spinal cord ependymoma and hemangioblastoma.

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